Linear and whorled nevoid hypermelanosis
Alternate names[edit | edit source]
LWNH; Nevoid hypermelanosis, linear and whorled; Linear and whorled hypermelanosis; Zosteriform lentiginous nevus; Zebra-like hyperpigmentation; Zosteriform hyperpigmentation; Reticulate hyperpigmentation of Iijima
Definition[edit | edit source]
Linear and whorled nevoid hypermelanosis (LWNH) is a rare benign condition affecting skin pigment occurring in newborns or early infancy.
Epidemiology[edit | edit source]
- It is unknown how many people have linear and whorled nevoid hypermelanosis.
- Most of the information on this condition comes from cases described in the medical literature.
Cause[edit | edit source]
- The exact cause of linear and whorled nevoid hypermelanosis (LWNH) is not known.
- It is thought to result from a genetic change called mosaicism, that occurs early in the development of an embryo.
- People with mosaicism have two sets of cells each with a different genetic makeup.
- Several chromosome abnormalities have been reported in affected individuals.
- People with LWNH who have an underlying chromosome abnormality are more likely to have symptoms in addition to those that affect the skin.
Inheritance[edit | edit source]
- Linear and whorled nevoid hypermelanosis (LWNH) generally occurs by chance in people with no family history (sporadically).
- A few rare cases of LWHN in more than one member of a family have been described.
Signs and symptoms[edit | edit source]
Symptoms may include:
- Swirling streaks of darkened (hyperpigmented) skin
- Developmental delay
- Asymmetric growth
- Cardiovascular defects
- The dark skin streaks appear in early infancy and are generally found on the trunk and limbs.
- The face, palms of the hand, and soles of the feet are not generally affected.
- The hyperpigmentation may get progress for a few years, and then become stable.
- In some people, the darkened skin streaks fade with time.
- Most people with LWNH have no medical problems related to their condition.
Diagnosis[edit | edit source]
It is diagnosed based on the symptoms.
Treatment[edit | edit source]
- There is no specific treatment for linear and whorled nevoid hypermelanosis (LWNH).
- It is considered a mild condition and no medical treatment may be necessary.
- Methods to lighten the skin including chemical peels and laser treatments may be helpful.
- Due to the possible association with chromosome abnormalities and other conditions, people with LWNH should be evaluated for developmental delays, growth delays, skeletal abnormalities, and congenital heart defects.
- One specialist who might be involved in the care of someone with LWNH is a dermatologist.
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